spinal chord
DESCRIPTION
Spinal chord. Spinal cord & Spinal Nerves. The spinal cord (45cm long) is part of the central nervous system and is connected to the brain Extends from the foramen magnum to the first lumbar vertebra (L1). - PowerPoint PPT PresentationTRANSCRIPT
Spinal chord
Spinal cord & Spinal Nerves
The spinal cord (45cm long) is part of the central nervous system and is connected to the brain
Extends from the foramen magnum to the first lumbar vertebra (L1).
The spinal cord is the main pathway for information connecting the brain and peripheral nervous system.
31 pairs of spinal nerves
Spinal Cord Protection
Bony Canal- vertebral canal
Connective tissue- meninges
Cushion of cerebrospinal fluid (CSF)- CSF acts as a shock absorber
Meninges
The meninges surround, protect, & stabilize the CNS
Consist of 3 layers DURAMATER ARACHNOID PIAMATER
Meninges
Epidural space- a space between bony canal & dura mater
Subdural space- A space between the dura mater & arachnoid mater- contains interstitial fluiid
Subarachnoid space- contains CSF fluid
Internal Anatomy of Spinal cord
Gray & white mater H- shaped gray
matter (central zone): dorsal & ventral horns
White matter (outer zone): - arrange in columns
Spinal Cord levels
The greatest amount of grey matter (cells) is largest in the
spinal segments of the cervical and lumbosacral enlargements
White mater (regions)
Anterior white column
Posterior white column
Lateral white column
Functions of spinal cord
Spinal cord reflexes: A reflex is a fast , involuntary, unplanned sequence of actions occur in response to a particular stimulus
Integration of information highway for upward and downward travel
of sensory and motor information
Classical spinal cord syndromesLesioned structures Causes
Complete sectionMielitisTrauma
Hemisection Brown-Séquard
Central structuresSyringomyeliaHydromyeliaTumor
Posterior columns and horns Tabes dorsalis
Posterior columns and lateral columns B12 deficiency
Classical spinal cord syndromesLesioned structures Causes
Dorsal columns, pyramidal tracts, spinocerebellar tract Spinocerebellar degeneration
Pyramidal tracts, anterior hornsALS
Anterior horn Spinal muscle atrophy,poliomyelitis
Pyramidal direct and crossed tracts Familial spastic paraparesis
Dorsal 1/3 of the spineOcclusion of the posterior spinal arteries
Anterior 2/3 of the spine Anterior spinal artery occlusion
Traumatic Spinal Cord Disease
10,000 new spinal cord injuries per year MVA, sports injuries the most common Victims under 30 yrs old, male>>females Fx/dislocation of vertabrae most likely to
occur at: C5,6 T12, L1 C1,2
Tumors
Metastatic or primary Extramedullary
Extradural - most common Bony - breast, prostate
Intradural - very rare Meninges - meningioma Nerve root - schwannoma
Intramedullary - very rare Metastatic Primary - astrocytoma or ependymoma
Centromedular formations
Intramedular tumors
Anterolateral column compression lead to sensory (thermoalgesic) loss that extends lower as the tumor grows
Lateral compression
Sensory disturbance appears first in the sacrate dermatoma; as the tumor grows, the sensory problems ascend upwards to the level of the lesion
Transverse myelitis
Inflammation of the spinal cord Post-infectious Post-vaccinial Multiple sclerosis
Pain at level of lesion may preceed onset of weakness/sensory change/b&b disturbance
Spinal tap may help with diagnosis
Infections Involving the Spinal Cord Polio
only the anterior horn cells are infected Tabes dorsalis
dorsal root ganglia and dorsal columns are involved tertiary syphillis sensory ataxia, “lightening pains”
HIV myelopathy mimics B12 deficiency
HTLV-1 myelopathy - tropical spastic paraparesis
Multiple Sclerosis
Demyelination is the underlying pathology Cord disease can be presenting feature of MS or
occur at any time during the course of the disease
Lesion can be at any level of the cord Patchy Transverse
Devic’s syndrome or myelitis optica Transverse myelitis with optic neuritis
Vascular Diseases of the Spinal Cord
Infarcts Anterior spinal artery infarct
from atherosclerosis, during surgery in which the aorta is clamped, dissecting aortic aneurysm
less often, chronic meningitis or following trauma
posterior columns preserved (JPS, vib)
weakness (CST) and pain/temperature loss (spinothalamic tracts)
Artery of Adamkiewicz at T10-11 Watershed area
upper thoracic
Vascular Diseases of the Spinal Cord, cont Arteriovenous malformation (AVM)
and venous angiomas Both occur in primarily the thoracic
cord May present either acutely, subacutely
or chronically (act as a compressive lesion)
Can cause recurrent symptoms If they bleed
Associated with pain and bloody CSF Notoriously difficult to diagnose
Hematoma - trauma, occasionally tumor
Other Diseases of the Spinal Cord Hereditary spastic paraparesis
Usually autosomal dominant Infectious process of the vertabrae
TB, bacterial Herniated disc with cord
compression Most herniated discs are lateral and
only compress a nerve root Degenerative disease of the
vertabrae Cervical spondylosis with a myelopathy Spinal stenosis
Brown Sequard Syndrome Cord hemisection Trauma or tumor Dissociated sensory loss
loss of pain and temperature contralateral to lesion, one or 2 levels below
crossing of spinothalamic tracts 1-2 segments above where they enter
loss of vibration/proprioception ipsilateral to the lesion
these pathways cross at the level of the brainstem
Weakness and UMN findings ipsilateral to lesion
Syringomyelia Fluid filled cavitation in the center
of the cord Cervical cord most common site
Loss of pain and temperature related to the crossing fibers occurs early
cape like sensory loss Weakness of muscles in arms
with atrophy and hyporeflexia (AHC)
Later - CST involvement with brisk reflexes in the legs, spasticity, and weakness
May occur as a late sequelae to trauma
Can see in association with Arnold Chiari malformation
Syringomyelia
Conus Medullaris vs. Cauda Equina Lesion
Finding Conus CE
Motor Symmetric AsymmetricSensory loss Saddle SaddlePain Uncommon CommonReflexes Increased/ Decreased
decreasedBowel/bladder Common possible
B12 Deficiency
Subacute combined degeneration of the cord
B12 deficiency malabsorption of B12 secondary to pernicious
anemia or surgery insufficient dietary intake - vegan
Posterior columns and CST involvement with a superimposed peripheral neuropathy
Friedreich’s disease
Degeneration of the long spinal
pathways (both ascending and
descending) and the peripheral nerve
fibers
Clinical presentation Gait abnormalities Ataxia Speech disorders Nistagmus Hyporeflexia Trophic changes
High plantar arches (pes cavus deformity of the foot)
Sensory disturbance Babinski sign
Clinical presentation Symptoms typically begin
sometime between the ages of 5 to 15 years,
scoliosis Diabetes (about 20% of people
with Friedreich's ataxia develop carbohydrate intolerance and 10% develop diabetes mellitus)
Heart disorders (e.g., atrial fibrillation, and resultant tachycardia (fast heart rate) and hypertrophic cardiomyopathy )
These symptoms are slow and progressive. Long-term observation shows that many patients reach a plateau in symptoms in the patient's early adulthood.
Genetics
Autosomal recesive disorder Mutation of frataxin gene (9q13-q21) Long arm of cromosome 9 Prevalence 1 : 50.000 Genetic counselling!
Treatment
Physical therapy Nerve and muscle trophic drugs Treatment of complications Treatment of heart disorders
Lateral Amiotrophic Sclerosis
Lateral Amiotrophic Sclerosis (ALS)
Degenerative disease of the central nervous system
Degeneration and death of motor neurons, both in the cortex and in the spine
Mean survival time is 3 to 4 years from diagnosis
USA – “Lou Gehrig’s disease”
ALS – clinical picture progressing motor deficit Signs related both to upper
and to lower motor neuron damage
starting age is around 60 years (20-80 years) Involvement of any part of the
CNS Either as UMN damage or as
LMN damage
SLA – clinical picture Muscle weakness
In 60-85% of pts starts in the limbs and in 15-40% starts with bulbar signs
Atrophy Stiffness Cramps Fasciculations;
Highly suggestive for ALS if largelly distributed and accompanied by UMN damage siogns
SLA – clinical picture Respiratory Insufficiency is seldom present
from the start Dispnea, asteny, weak cough, predisposition for
developping pneumonia ant athelectasies With time, LMN signs may overcome all of the UMN
signs Brainstem damage signs
disphagia disarthrie sialorrhea (reduction of swallowing) laringospasm Lately in the course of disease oculomotor problems
Criteriile El Escorial revăzute pentru diagnosticul sclerozei laterale amiotrofice
Criteriul de diagnostic Semne necesare
SLA sigură Semne ale NMC şi ale NMP în cel puţin 3 din 6 regiuni
SLA familială sigură Semne de afectare ale NMC şi NMP în o regiune (plus identificarea mutaţiei asociate prin procedee de laborator)
SLA probabilă Semne de afectare a NMC şi NMP în 2 din 6 regiuni (unele semne de NMC fiind rostral situate faţă de cele de NMP)
SLA probabilă, susţinută de testele de laborator
Semne de afectare ale NMC si NMP în o regiune (sau semne de laborator ale afectării NMC într-una sau mai multe regiuni plus proba EMG a denervării acute în 2 sau mai mulţi muşchi situaţi la nivelul a două sau mai multe membre)
SLA posibilă Semne de afectare ale NMC şi NMP în o regiune
ALS
EMG criteria for ALS (Lambert): Normal sensory conduction velocity Motor conduction velocities normal or nearly normal,
depending of the muscke waisting Fibrilation and fasciculations Increased amplitude and duration of action potentials
EMG is only accesory to diagnosis in ALS
Evaluation and follow up
Global evaluation scales for ALS: Scoring based on subjective and clinical data
Muscle force measuring Evaluation of quality of life
Treatment
Maintaining a good quality of life as long as possible
Prolongation of active life Prolongation of life expectancy
Drug Trials
Negative results Vitamin E TCH346 Celebrex Creatine Indinavir Topiramate(topamax) Neurontin Minocycline
Other drugs Lithium Arimoclomol Ceftriaxone Manganoporphirin
Medical ManagementRiluzole The only FDA approved drug for ALS
treatment Approved for use in the US, Europe- 1996
and Canada –2000 Safe, fairly well tolerated Moderate efficacy Expensive ($10,000/year !!)
Prolongs survival by 3-4 months, Cochran review 2005
Side effects include fatigue, nausea, diarrhoea and liver toxicity
Muscle weakness
Aerobics, exercises to improve flexibility, force As the disease progresses the rehabilitation
programme has to be adapted to possibilities Establish safe exercises without overexertion
Maintaining independence Assistive devices (wheel chair, cruches, walker,
voice command, lifts, large doors, adapted vehicles)
Symptomatic Management
Important part of ALS treatment Several recent advances for symptom
management Increase in survival and quality of life in
patients seen in ALS centers
Symptomatic Management Emotional lability (Pseudobulbar affect) Spasticity Cramps and Fasciculations
Difficult to treat generally -Magnesium –Slow Mag -Tonic water (quinine) -Chamomile tea ? -Low dose baclofen -Low dose benzodiazepines
Treatment of Pain Can occur at any stage in the disease Neuropathic, Musckulosketetal, immobility Combination of drugs NSAIDS, Gabapentin, Pregabalin, TCAs Severe Pain – Narcotics
Symptomatic Management
Treatment of Fatigue Amantidine – dose 100 mg Po twice or three
times daily Modafinil (Provigil)- 100 mg 1-2 times a day Treatment of other underlying causes ( thyroid dysfunction, anemia etc.)
Insomnia Bowel and Bladder Dysfunction
ALS Management Thick Mucus Production
Increase Hydration Guiafenesin (robitussin) liquid or pill form Propananol ? Suctioning and cough assist device
Management of Dysphagia
Sialorrhea Management Glycopyrrolate (Robunil)- 1-2 mg every 4 hours Benztropine, Amitriptyline Transdermal hyoscine patch Topical atropine drops - 1% every 4 hours Botox or Myobloc injection into the salivary glands Radiation therapy – if very severe (AAN Practice Parameter- R. G Miller and Colleagues, revised Nov, 2006)
Management of Respiratory Failure FVC checks every 3 months in clinic BIPAP if FCV < 50 PPV Invasive ventilation/Tracheostomy when FVC <
30 PPV Treating underlying infection Management of patient anxiety Early signs – sleep problems, morning
headache, ortopnea, shallow breath, speech problems, sigh
Palliative Care
Palliative services involved early Hospice for patients with end stage ALS Comfort care and pain management Early Decision making Advanced Directives